Is there a role for steroids in the treatment of Guillain-Barré Syndrome (GBS)?

No Role for Steroids in the Treatment of Guillain-Barré Syndrome

Steroids are not recommended for the treatment of Guillain-Barré Syndrome (GBS) as multiple randomized controlled trials have shown no benefit and potential harm with their use. 1

Evidence Against Steroid Use in GBS

The evidence against steroid use in GBS is strong and consistent:

• Multiple randomized controlled trials have demonstrated that corticosteroids provide no significant benefit in GBS and may even have negative effects 1, 2
• Eight randomized controlled trials specifically showed no significant benefit of corticosteroids for GBS, with oral corticosteroids actually showing negative effects on outcomes 1
• A Cochrane systematic review concluded that "corticosteroids should not be used in the treatment of Guillain-Barré syndrome" 2

Recommended First-Line Treatments for GBS

The standard first-line treatments for GBS with proven efficacy are:

1. Intravenous Immunoglobulin (IVIg):

   • Recommended dosage: 0.4 g/kg body weight daily for 5 days 1, 3
   • Indicated for patients within 2-4 weeks after onset of weakness who are unable to walk unaided 3
   • Generally preferred over plasma exchange due to easier administration and wider availability 1

2. Plasma Exchange (PE):

   • Recommended protocol: 200-250 ml plasma/kg body weight in five sessions 1
   • Indicated for patients within 4 weeks after onset of weakness who are unable to walk unaided 3
   • Equally effective as IVIg but may have higher discontinuation rates 1

Treatment Algorithm for GBS

1. Assess severity and timing:

   • If patient is within 2-4 weeks of symptom onset and unable to walk unaided, proceed with immunotherapy
   • If patient can walk independently (mild GBS), close monitoring may be appropriate

2. Choose treatment modality:

   • First choice: IVIg 0.4 g/kg/day for 5 consecutive days
   • Alternative: Plasma exchange (if IVIg unavailable or contraindicated)

3. Monitor for respiratory compromise:

   • Use the Erasmus GBS Respiratory Insufficiency Score (EGRIS) to identify patients at risk for respiratory failure 1
   • Consider ICU admission for patients with evolving respiratory distress, severe autonomic dysfunction, severe swallowing dysfunction, or rapid progression of weakness

4. Do NOT add steroids:

   • Neither oral nor intravenous corticosteroids have shown benefit 1
   • Combination therapy of plasma exchange followed by IVIg is also not recommended 1

Special Considerations

• GBS variants: Patients with Miller Fisher Syndrome (MFS) tend to have a milder course and may not require treatment, but should be monitored closely 1
• Treatment-related fluctuations: About 10% of GBS patients experience secondary deterioration within 8 weeks after starting IVIg, which may require repeated treatment 4
• Chronic progression: Consider changing diagnosis to acute-onset chronic inflammatory demyelinating polyradiculoneuropathy (A-CIDP) if progression continues after 8 weeks from onset (occurs in about 5% of patients initially diagnosed with GBS) 3, 4

Management of Complications

• Pain management: Consider gabapentinoids, tricyclic antidepressants, or carbamazepine for neuropathic pain 3
• Respiratory support: Up to 22% of GBS patients require mechanical ventilation within the first week of admission 1
• Autonomic dysfunction: Monitor for cardiac arrhythmias and blood pressure fluctuations 1

Despite the lack of benefit from steroids in idiopathic GBS, it's worth noting that in the specific context of immune checkpoint inhibitor-related GBS, a trial of methylprednisolone 1-2 mg/kg may be reasonable 1, but this represents a distinct clinical entity from typical GBS.